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A 60-year-old woman is evaluated in the emergency department for hematuria and moderate abdominal pain. She has mild hypertension treated with a β-blocker but is otherwise healthy. Family history is negative for renal disease, kidney stones, and hypertension.

On examination, her blood pressure is 152/95 mm Hg, pulse rate 90/min, respiration rate 15/min, temperature 37 ºC (98.6 ºF). There is a grade 3/6 late systolic murmur preceded by multiple clicks; the murmur is best heard at the cardiac apex. There is a moderately tender abdominal 7- x 8-cm mass in the left flank extending to the umbilicus. There is a suggestion of a right-sided abdominal mass, as well. There is no costovertebral angle tenderness. Rectal examination is normal; stool is negative for occult blood.

Hemoglobin is 14 mg/dL, blood urea nitrogen is 50 mg/dL, and serum creatinine is 3.3 mg/dL. Her urine appears grossly bloody with too many erythrocytes to count. A urine culture is negative.

This condition causing the patient™s findings is associated with an increased risk for which of the following?

( A ) Ovarian cyst
( B ) Cerebellar hemangioblastoma
( C ) Adrenal adenoma
( D ) Hepatic cyst
( E ) Abdominal aortic aneurysm

ddd

Autosomal dominant polycystic kidney disease (ADPKD) occurs in one in every 400 to 1000 live births. Although affected patients usually have a family history of renal disease, in 25% to 40% of new cases the family history is negative, either because of a new mutation or because the disease had been clinically silent in affected family members, who died of other causes before the diagnosis could be made. The clinical presentation in this patient, gross hematuria, bilateral abdominal masses (which are the enlarged, cystic kidneys), and renal failure is typical. The acute abdominal pain is most likely secondary to rupture of a renal cyst. This patient™s cardiac examination is consistent with mitral valve prolapse, which has a higher incidence in patients with ADPKD.

The incidence of hepatic cysts in ADPKD increases with age, and is approximately 40% in patients older than 60 years. Massive, symptomatic cysts are almost exclusively seen in females, perhaps because of the effect of female steroid hormones on cyst growth. Most ADPKD patients with hepatic cysts have preserved liver function and are not symptomatic from these cysts.

Up to 10% of patients with ADPKD have cerebral aneurysms. The incidence of a cerebral aneurysm is higher in patients with polycystic kidney disease who have a family history of cerebral aneurysms (~25%), and aneurysm rupture is more likely in a patient with ADPKD. The risk of rupture is greatest in those with 10-mm aneurysms or larger, previous aneurysms, or a history of uncontrolled hypertension. An increased incidence of cerebellar hemangioblastoma has not been reported.

rt kaps!

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showman and kaps....good night.thanks...

it is till 5 pm US time... thank you guys .. come back and post questions again

it 2 am in india

hey showman - kaps mutual admiration club others lost the chance of answering

thanx for nice questions and gnite to you both... bbye

good nite guest and pubmed